AB0522 EARLY DAMAGE AS MEASURED BY THE DAMAGE INDEX FOR ANTIPHOSPHOLIPID SYNDROME (DIAPS) IS NOT A PREDICTOR OF MORTALITY IN THROMBOTIC ANTIPHOSPHOLIPID SYNDROME
Authors: P. Gaspar, F. Farinha, Z. Sayar, M. Efthymiou, H. Cohen, D. Isenberg
Journal: Annals of the Rheumatic Diseases (2022)

Background Antiphospholipid syndrome (APS) patients experience damage accrual (1) but correlation with mortality is rarely reported. In systemic lupus erythematosus (SLE), early damage accrued in the first year of disease is a predictor of mortality (2). Objectives To determine whether damage accrued within the first year after APS onset as measured by the Damage Index for APS (DIAPS) predicts mortality. Methods Single-centre retrospective analysis of thrombotic APS patients (2006 Sydney criteria). The disease onset was determined as the time the first thrombotic event related to APS was diagnosed. Annual DIAPS assessments were determined for each patient. Early damage was considered to be a score ≥1 at the initial assessment (six months). DIAPS is shown as median (interquartile range [IQR]). Survival was analysed by the Kaplan-Meier method. Cox regression analysis was performed to investigate predictors of mortality. Results A total of 197 patients (71.1% female; 65.9% primary APS; 72.4% Caucasian) with a median age at APS onset of 40 years (IQR 51 - 28) were followed for up to 43 years (median 10 years). Cumulative damage developed in 143 (73.6%) patients with a median DIAPS of 1 (IQR 2 - 0) at the last visit. Early damage was present in 69 (35.0%) patients. Patients with early damage had similar sex distribution ( p =0.733), age at disease onset ( p =0.526) and median time of follow-up ( p =0.181) comparing to those without early damage. Secondary APS (SAPS) (odds ratio [OR] 1.89, 95% CI 1.03 - 3.48, p =0.041) and having a first arterial event (OR 7.24, 95% CI 3.74 - 14.03, p &lt;0.001) were associated with early damage. Twenty-three patients (11.7%) died. SAPS ( p =0.009), male sex ( p =0.008) and age at onset ≥40years ( p =0.001) were risk factors for death (Table 1). Early damage was not associated with death (hazard ratio [HR] 1.65, 95% CI 0.73 - 3.78, p =0.231; Figure 1: survival curve) even after adjusting individually for APS category (secondary, p =0.446), sex (male, p =0.374), age at onset ≥40years ( p =0.115) and type of event (arterial, p =0.555). Table 1. Predictors of mortality. Crude analysis Adjusted analysis † HR (95% CI) p-value HR (95% CI) p-value Male sex 3.14 (1.35 – 7.33) 0.008 1.46 (0.63 – 3.36) 0.374 Age at APS onset ≥ 40 years 5.34 (1.9 – 14.53) 0.001 1.95 (0.85 – 4.50) 0.115 Caucasian 0.95 (0.35 – 2.57) 0.918 1.61 (0.70 – 3.70) 0.257 Secondary APS 3.07 (1.32 – 7.12) 0.009 1.38 (0.59 – 3.20) 0.446 First event (arterial) 1.85 (0.79 – 4.27) 0.151 1.32 (0.53 – 3.32) 0.555 Early damage 1.65 (0.73 – 3.78, 0.231 - - † In this column, we present the Early Damage hazard ratio and 95% confidence intervals for death adjusted for the variable in each row. Statistically significant values are presented in bold. APS, antiphospholipid syndrome; CI, confidence interval; DIAPS, Damage Index for Antiphospholipid Syndrome; HR, hazard ratio. Figure 1. Survival probability in patients with and without early damage. Conclusion Unlike SLE, early damage accrual accessed by DIAPS is not a predictor of mortality regardless of the nature of the thrombotic event, gender, APS category and age at APS onset. References [1]GRIKA EP, ZIAKAS PD, ZINTZARAS E, MOUTSOPOULOS HM, VLACHOYIANNOPOULOS PG: Morbidity, Mortality, and Organ Damage in Patients with Antiphospholipid Syndrome. J Rheumatol. 2012;39(3):516–23. [2]RAHMAN P, GLADMAN DD, UROWITZ MB, HALLETT D, TAM LS: Early damage as measured by the SLICC/ACR damage index is a predictor of mortality in systemic lupus erythematosus. Lupus . 2001;10(2):93–6. Disclosure of Interests Pedro Gaspar: None declared, Filipa Farinha: None declared, Zara Sayar: None declared, Maria Efthymiou: None declared, Hannah Cohen Speakers bureau: Honoraria for lectures from Bayer Healthcare (outside the submitted work)., Consultant of: Consultancy fees from UCB Biopharma paid to University College London Hospitals Charity (outside the submitted work)., David Isenberg: None declared